Periodic fever, aphthous stomatitis, pharyngitis and adenitis

What causes periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA)?

The underlying cause of PFAPA is not yet known. While it appears to involve inflammatory processes, it is not clear why episodes develop. No gene or infection has been found to be responsible for causing PFAPA and it is not contagious.

Last updated on 05-01-20

How is periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) diagnosed?

There are no laboratory tests or imaging procedures specific to the diagnosis of PFAPA. This condition is clinically diagnosed in individuals who have a history of 3 or more episodes of fevers that last up to 5 days and recur at regular intervals without other evidence of acute illness. Pharyngitis (sore throat) plus adenopathy (swollen lymph nodes) or aphthous ulcers (canker sores) are also noted. Blood tests like white blood cell count, C-reactive protein, and erythrocyte sedimentation rate (ESR) are often elevated during an acute attack (but normal between attacks). It is important to rule out other conditions that may present with similar symptoms (for example, strep throat). The dramatic response to treatment can help to confirm the diagnosis.

Last updated on 05-01-20

**How might periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis be treated?**

Treatment options that have been successful in improving symptoms of this condition include: oral steroids (prednisone or prednisolone), tonsillectomy with adenoidectomy and cimetidine.

Last updated on 05-01-20

Selected Full-Text Journal Articles

Free full text - PFAPA Wong

Wong KK, Finlay JC, Moxham JP. Role of Tonsillectomy in PFAPA Syndrome. Otolaryngol Head Neck Surg. 2008 Jan;134(1):16-9.

Last updated on 04-27-20

Free full text - PFAPA Licameli

Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2008 Feb;134(2):136-40.

Last updated on 04-27-20

Free full text - PFAPA Padeh

Last updated on 04-27-20

Where To Start

MerckManual - Periodic fever syndrome

The Merck Maual provides information on periodic fever syndromes in general. The Merck Manuals are a series of healthcare books for medical professionals and consumers.

Last updated on 04-27-20

Name: FMF & AID Global Association Schüracherstrasse 25a 8306 Brüttisellen
Switzerland
Phone: +41 76 415 4010 Email: info@fmfandaid.org Url: https://www.fmfandaid.org/
Periodic Fever Syndrome Cleveland Clinic Web site. 2015; Reference Link PFAPA Autoinflammatory Alliance. 2014; Reference Link Stephen E. Goldfinger. PFAPA Syndrome Merck Manual Professional Version. February 2013; Reference Link

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